Institutional experience with cranial vault encephaloceles

There is scant literature regarding the long-term outcome in patients with cranial vault encephaloceles, and what literature there is may underestimate long-term deficits. The goal of this study was to address this lack of information.

The authors performed a retrospective chart review of cranial vault encephaloceles performed at our institution between 1989 and 2003. Fifty-two total patients were identified and 44 of these cases were reviewed. Additionally, 34 of the 44 patients were contacted and given an outcome survey (Hydrocephalus Outcome Questionnarie [HOQ]) to evaluate physical, emotional, cognitive, and overall health outcomes.

The mean age for patients in this cohort was 9.6 years (range 4-17 years) and the mean follow-up time was 9.2 years. There was an equal sex distribution and there were no deaths. Hydrocephalus was found in 60% of occipital and 14% of frontal encephaloceles, and epilepsy was confirmed in 17% of occipital and 7% of frontal lesions. Outcome assessments performed using the HOQ showed that 50% of the patients with occipital encephaloceles had overall HOQ health scores of 0.5 or less and 55% had HOQ cognitive scores of 0.3 or less, compared with 0% of patients in both categories who had frontal encephaloceles. It was also found that the presence of hydrocephalus and epilepsy independently and significantly lowered the overall health scores.

Occipital encephaloceles carry a worse prognosis than frontal encephaloceles, with higher rates of hydrocephalus and seizure. Based on this study, the presence of hydrocephalus and epilepsy are significant additive adverse prognostic factors. Approximately half of the patients with occipital encephaloceles will be severely debilitated and will probably be unable to live and function independently in society. These data may be useful to clinicians in counseling patients and predicting long-term outcome following repair of cranial vault encephaloceles.